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259
Evaluation of Surgical Procedures for Cyanotic Congenital Heart Disease by Using MR Imaging
Barbara
A. Kersting-Sommerhoffi Klaus C. Seelos1 Christian Hardy2 Chisato Kondo1 Sarah S. Higgins2 Charles B. Higgins1
ECG-gated MR imaging has been shown to be effective for the diagnosis of congenital disease. In this study, we assessed its role in the postoperative evaluation of surgical procedures in patients with complex congenital heart disease. MR images of 26 patients with Rastelli (five), Fontan (three), Senning (three), Damus (one), Jatene (eight), Waterston (four), and Potts (two) procedures were evaluated retrospectively. The accuracy of MR imaging was compared with that of angiography in 20 patients. The surgical anastomoses were identified in all patients. Patency, atresia, or hypoplasia of central pulmonary arteries and postoperative complications (focal stenoses of pulmonary arteries, thrombosed conduit, peri-conduit abscess) were shown. Narrowing of the right ventricular outflow tract and focal compression of the proximal pulmonary arteries were recognized as specific complications of the Jatene procedure. MR imaging appears to be effective in the postoperative evaluation of surgical heart
procedures
used for congenital
heart disease.
It should
to repeated catheterization and angiography children with complex congenital heart disease.
AJR 155:259-266,
Aeceived February vision April 19, 1990. I
Department
16,
1990;
of Aadiology
accepted
(L308),
after reBox
0628,
University of California, San Francisco, Medical Center, San Francisco, CA 94143. Address reprint requests to C. B. Higgins. 2 Oakland Children’s Hospital, 51 05 Dover St., Oakland,
CA 94609.
0361 -803x/90/1 552-0259 Roentgen Ray Society
© American
August
be considered
for the
as an alternative
postoperative
examination
of
1990
Previous studies have established spin-echo MR imaging as an effective, noninvasive method for evaluating congenital disease of the heart [1 -7] and the great arteries [8-1 1]. When compared with echocardiography, one of the major advantages of MR imaging is its usefulness for evaluating great vessel abnormalities. Because a number of surgical procedures used in the treatment of cyanotic heart disease involve the great arteries or cardiovascular structures superior to the heart, MR imaging may be particularly useful in the assessment of these structures. Indeed, previous reports have shown the effectiveness of MR imaging for the evaluation of systemic to pulmonary shunts [12, 13]. The arterial switch (Jatene) procedure [14, 15], the Damus-Kaye-Stansel (Damus) procedure [16, 17], and the Rastelli procedure [1 8] are other surgical procedures involving structures above the base of the heart. The usefulness of MR imaging for evaluating these procedures has not yet been reported. The purposes of the current study were (1 ) to assess the effectiveness of spinecho MR imaging in the postoperative examination of patients with surgical procedures in the supracardiac region for the treatment of cyanotic congenital heart disease; (2) to compare the accuracy of MR imaging with that of angiography for identifying complications of the surgical procedures, including occlusion, stenosis, and aneurysm formation of conduits and anastomoses; and (3) to compare MR imaging with angiography for defining the status of the pulmonary arteries distal to various anastomoses and conduits. Subjects Twenty-six procedures
and Methods patients
(Rastelli,
with
congenital
five; Fontan, three;
heart
disease
Senning,
three;
and
palliative
Damus,
one;
or
corrective
Jatene,
eight;
surgical Water-
260
KERSTING-SOMMERHOFF
ston,
four;
imaging.
and
two)
description
underwent
ECG-gated,
of the surgical
procedures
spin-echo and
their
is given
to 28 years
patients
who
congenital
Images ing
were
were
magnet
ECG
acquired
was
in the transverse were
surgical
by using MT/S,
an echo
the AR interval images
after
a cryogenic
Milpitas,
(General
delay
applied
time
plane
in all patients; were
CA)
Electric (TE)
and thus depended acquired
procedures
for
0.35-T or
MR images reviewers
Signa,
of 25-30 the repetition
on the patient’s
obtained
depending
in all patients; on the
type
were time
anastomosis; WI).
obtained.
or coronal procedure
(in 17 and nine patients, of both
respectively). Images with a slice thickness 5 and 10 mm were obtained in 19 patients. In the last patients
TABLE
1: Surgical Procedure
Rastelli
did
of
not
retrospectively the
know
general the
by three reviewers.
diagnosis
results
of
other
definition
of the
central
pulmonary
Operative
Description from
Corrected
Lesion
RV to MPA
Pulmonary
atre-
sia, severe pulmonary stenosis Fontan
Conduit
or direct
anasto-
Tricuspid atresia, severe tricuspid ste-
of right atrial ap-
mosis pendage
to MPA
nosis, Senning
Surgically
created
baffle
teries
nary atrial chambers End-to-side anastomosis of MPA to ascending
Ao; shunt
Severe stenosis of LVOT; ob-
from Ao to
structing VSD in tncuspid atresia Transposition of great antenies
MPA
Jatene
Transsection
of great
ar-
teries above sinuses and switching of arteries to opposite
nuses:
Potts
hypo-
plastic RV Transposition of great ar-
dividing the atrium into systemic and pulmoDamus
si-
reimplantation
of coronary arteries from aortic sinuses to the switched ascending Ao Side-to-side anastomosis
of descending left PA
Pulmonary
Ao to
nosis
ste-
(tetnal-
ogy
of Fallot), pulmonary
atnesia, cuspid sia Waterston
Side-to-side
of ascending right
tnatne-
Pulmonary
anastomosis
Ao to
nosis
ogy
PA
ste-
(tetnalof Fallot),
pulmonary atnesia, tncuspid sia
=
Note.-RV ventricular
= right ventricle; MPA = main pulmonary septal defect; PA = pulmonary artery.
the
imaging
The
surgical studies
features: demof the site of
arteries;
Procedures
Conduit
and
and
identi-
fication of postoperative complications at valvar, anastomotic, and arterial levels. Successful studies had to meet the following criteria: depiction of the shunt or baffle on at least two images or in two different planes and direct visualization of the systemic-pulmonary arterial anast#{243}mosis. Patency of a shunt or conduit was determined by the absence of signal in the vessel lumen. The MR results were compared with the findings at surgery and angiography. Twenty of the patients had angiography to corroborate MR findings. In these 20 patients, MR and angiographic findings were
1 .5-T
(TR) equaled
sagittal
but
aware
or angiography). The MR images were evaluated for the following onstration of the shunt, conduit, or baffle; depiction
heart rate. Images
of surgical
1990
(echocardiography
superconductMilwaukee,
msec
were evaluated
were
procedure,
complex
a cryogenic
August
patients.
disease.
magnet
with gating
imaged heart
(Diasonics
superconducting Images
in Table
(mean,
cyanotic
AJR:155,
imaged who had had Jatene procedures, images of 3-mm thickness were acquired. In seven older children and adults, the slice thickness was 1 0 mm. Angiographic studies were available in 20 of the 26
MR major
1 . Patients ranged in age from 6 months 7 years). Twelve children were 3 years old or younger. Children under 6 years old were sedated 30 mm before the examination with 100 mg/kg chloral hydrate (maximum total dose, 1 500 mg) orally or rectally, or nembutal 5 mg/kg (maximum dose, 1 00 mg) intramuscularly. The subjects of the study were consecutive applications
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Potts,
A brief
ET AL.
artery;
Ao
=
aorta;
LVOT
=
atne-
left ventricular
outflow
tract;
VSD
AJR:155,
August
MR OF CYANOTIC
1990
CONGENITAL
compared. In the remaining patients, echocandiognaphy and surgical findings were used to corroborate the findings of MR imaging.
HEART
261
DISEASE
the one remaining case, a diminutive pulmonary artery was found during autopsy. All other pulmonary arterial segments were found to be normal with both techniques.
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Results Demonstration
of Anastomoses
with MR
Complications
The shunts, conduits, or baffles were successfully imaged in all 26 patients. In all 26 cases, the anastomoses could be visualized directly on transverse images. Waterston, Potts, Fontan, and Damus procedures were best visualized on transverse images: images in the coronal plane generally did not provide important additional information. Rastelli and Jatene procedures were depicted on transverse and sagittal images; images in the sagittal plane were especially valuable for detecting postoperative complications (Fig. 1). In all but four cases, the surgical connection could be seen on more than one image; in these four cases, the slice thickness was 1 0 mm. The Rastelli, Senning, and Damus procedures were visualized longitudinally on images in the sagittal or coronal plane (Figs. 2 and 3).
Comparison Central
of MR and Angiography
Pulmonary
Arteries
Pulmonary arterial segments of 20 patients were analyzed and compared with angiographic findings, with consideration of the main, left, and right pulmonary arteries as separate segments (Table 2). Three right and two left pulmonary arteries were diagnosed as stenotic on both MR and angiography. Eight pulmonary arterial segments (one right, one left, six main) were not visualized with MR imaging or angiography. On review of the patients’ surgical or autopsy reports, these vessels proved to be atretic in seven of eight segments. In
Fig. 1.-Adjacent transverse MR images of a patient A, Aorta (A) is located posterior to main pulmonary
Table 3 summarizes the number of complications evaluated by MR and angiography and the number of complications identified by the two techniques. Complications were seen in all four patients who had undergone the Rastelli procedure. In two patients, narrowing of the conduit was identified; in the third, a thrombus obstructing the conduit was detected with MR imaging. One of these patients had a stenosed right pulmonary arterial segment also. The fourth patient, with clinical symptoms of endocarditis, had an intramural abscess within the right ventricular wall (infectious pseudoaneurysm) and surrounding the conduit. Two of these patients were studied with angiography, and the complications were detected in both. The complications in all four patients were confirmed during subsequent surgery. Four complications of Fontan procedures were seen by MR in two different patients. However, only two of the four complications were identified by angiography. Considerable right atrial enlargement, with the atrial septum bulging into the left atrium, was present in both patients. This enlargement compressed the entrance of the right pulmonary veins into the left atrium. A thrombus adhering to the atrial septum was shown with MR in one of these patients. The bulging septum and compression of the ostium of the right pulmonary vein was confirmed with angiography, but the thrombus was not identified. The other patient had a cardiac mass that was diagnosed with MR imaging as a partially thrombosed pseu-
with transposition of great arteries and Jatene procedure. artery (P) and compresses proximal right (RP) and left (LP) pulmonary
arteries (arrows
= focal
stenoses). B, Aortic root (AR) is dilated and right ventricular outflow tract (asterisk) is hypertrophied. C, Sagittal MR image of a different patient who had undergone same procedure. Note severe carotid artery, A = aorta, asterisk = RV outflow tract, RV = right ventricle, LV = left ventricle.
right ventricular
outflow
tract
stenosis
(black
arrow).
C =
262
KERSTING-SOMMERHOFF
ET AL.
AJR:155,
Fig.
2.-A-D,
August
1990
(A, B) and coronal of patient with malaligned
Transverse
(C, D) MR images
atrioventricular canal and subaortic stenosis treated with Damus procedure (end-to-side anastomosis of proximal main pulmonary artery
[P] and ascending
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aorta [A]). Wide communica-
two vessels is clearly shown in both planes (A, C). B depicts narrowing of left ventricular outflow tract (curved arrow) as well
tion
between
as left and right ventricular hypertrophy. Note pulmonary veins entering left atrium (LA). Small arrows on A are pulmonary valve leaflets. RV = right ventricle, LV = left ventricle, RA = right atrium, I = innominate artery, C = carotid artery.
doaneurysm measuring 6 cm in diameter and arising from the right ventricle (Fig. 4). The presence of a pseudoaneurysm was not diagnosed with angiography but was confirmed at subsequent surgery. MR imaging and angiography in a patient with the Senning procedure showed stenosis at the superior limit of the baffle, causing superior vena caval obstruction (Fig. 5). Another patient had pulmonary arterial hypertension after surgery. This was shown on MR images by enlarged pulmonary arteres and an elliptical configuration of the left ventricle, rather than the crescentic shape usually observed after this procedune. The ventricular septum in this patient was bowed toward the right ventricle, which is the reverse of the usual appearance in patients with transposition of the great arteries. In the patient with the Damus procedure, MR imaging identified a thrombus obstructing the left pulmonary artery (Fig. 2). This complication was not detected on angiography; the angiographic finding was considered as poor opacification of the left pulmonary artery for unknown reasons. The thrombus appeared as an area of high signal intensity completely obstructing the lumen of the left pulmonary artery. The thrombus was removed at subsequent surgery, and a follow-up
MR study showed no residual obstruction of the pulmonary artery. The eight patients with Jatene procedures had 1 5 morphologic abnormalities that were all shown with MR imaging (Table 1) (Fig. 1). Dilatation of the aortic root (three patients), supravalvar aortic stenosis (one patient), and narrowing of the right ventricular outflow tract (six patients) were depicted on MR images in all cases. These were confirmed with angiography in the four patients who had this study. The remainder had identical findings on echocardiography. Proximal narrowing of the right and left pulmonary arteries as they coursed around the repositioned aorta was identified in six patients on transverse MR images (Fig. 1). There was complete agreement between MR imaging and angiography with regard to the nine abnormalities evaluated by both techniques. No complications were detected in the six patients with Waterston or Potts shunt.
Discussion This study assessed the echo MR imaging to evaluate
capability of ECG-gated, spinsurgical procedures used in the
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AJR:155,
August
MR OF CYANOTIC
1990
CONGENITAL
HEART
263
DISEASE
Fig. 3.-A-D, Transverse (A-C) and sagittal (D) MR images of patient with truncus arteriosus and uncomplicated Rastelli procedure. On transverse images, conduit (C) can be followed from right ventricle (RV) to its entrance into main pulmonary artery (P). Conduit is depicted longitudinally on sagittal image. A = aorta, LA left atrium, RA = right atrium, LV = left ventricle, RP = right pulmonary artery, LP = left pulmonary artery.
, ,
i.;i; I”-.’
‘u..J
C TABLE
2: Status
of Pulmonary Right (n =
Nonmal Stenotic Atnetic Note-PA
=
Arterial
PA
D (n
Segments
Left PA (n = 20)
20)
= 60)
Main (n =
PA
20)
MR
Angio
MR
Angio
MR
Angio
16 3 1
16
17
17
14
14
0 6
0 6
pulmonary
artery.
3 1
2 1 Angio
2 1 =
angiography.
treatment of cyanotic congenital heart disease. In comparison with angiography, the results show that MR imaging can reliably depict the anatomy of the surgical procedures, postoperative complications, and the status of the central pulmonary arteries. MR imaging is as effective as angiography for the identification of these complications and of the status of the pulmonary arteries. The results suggest that MR imaging could be used to monitor size, patency, and eventually the growth of the pulmonary arteries under improved perfusion conditions after construction of systemic-pulmonary shunts. In an earlier study, Jacobstein et al. [1 2] reported the usefulness of ECG-gated MR for the evaluation of systemic-
pulmonary artery shunts in a study population that consisted mostly of patients with Blalock-Taussig and Glenn shunts. The current study focused on patients with Rastelli, Fontan, and Senning procedures, and two of the newer surgical techniques, namely Damus and Jatene procedures, for connection on palliation of cyanotic congenital heart disease. In patients who underwent a Rastelli procedure, MR imaging displayed the conduit between the right ventricle and the pulmonary artery (Fig. 3) in the transverse and sagittal planes. The sagittal plane was found to be the most effective for showing the proximal anastomosis to the right ventricle and the distal anastomosis to the pulmonary artery. Narrowing of the conduit, stenosed origins of the right or left pulmonary arteries, and pseudoaneurysm were complications depicted with MR imaging. Compression of the graft by the sternum or other structures in an excessively narrowed space anterior to the right ventricle can be detected on sagittal MR images. Demonstration of valvular obstruction is limited with spinecho MR imaging. The Fontan procedure creates an anastomosis between the right atrium and pulmonary artery; it can generally be done either directly between the right atrial appendage and pulmonary artery or with graft material between the right
KERSTING-SOMMERHOFF
264
TABLE
3: Complications:
Comparison
ET AL.
AJR:155,
1990
of MR and Angiography
MR Imaging
Angiognaphy
Procedure
Specific Complications No. Studied
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August
No. Identified
No. Studied
No. Identified
Rastelli
4
4
2
2
Fontan
4
4
4
2
Senning Damus
1 1
1 1
1 1
1 0
15
15
9
9
Narrowing of conduit (n = 2), thnombosed conduit (n = 1), peniconduit abscess (n = 1) Aneunysmal dilatation of RA (n = 2), RA thnombus (n = 1), RV pseudoaneunysm(n=1)
Jatene
Note.-AA
=
right atrium;
RV
=
right ventricle;
PA
=
pulmonary
artery;
AVOT
Baffle stenosis (n = 1) Thrombus in left PA (n = 1) Aortic dilatation (n = 3), supravalvular aortic stenosis (n = 1), narrowing of RVOT (n = 6), proximal PA stenosis (n = 6) =
right ventricular
outflow
tract.
Fig. 4.-A-C, Transverse MR images of patient with tricuspid atresia and Fontan procedure. Right atrium (RA) is markedly enlarged, with atrial septum (arrowhead) bulging toward left and compression of entrance of pulmonary veins (arrow) into left atrium (LA). Conduit (C) originates from right atrium and enters the pulmonary artery (P). Phase-encoded imaging indicated slow flowing blood within a partially thrombosed pseudoaneurysm (asterisk) anterior to right ventricle. A = aorta.
atrium and pulmonary artery [1 9, 20]. Infrequently a valve may be included in the anastomosis. One recent report has described the effectiveness of MR imaging for the postoperative evaluation of the Fontan procedure [21 ], showing patency of the anastomosis and the size of the central and hilar pulmonary arteries. Complications [22] of this operation indude conduit obstruction, residual atrial septal defects, and systemic venous hypertension. Residual septal defects, however, may not be recognized with MR imaging and require Doppler or color flow echocardiography or angiography for detection and quantification. In the current study, an unusual complication, a pseudoaneurysm surrounding the anastomo-
sis, was characterized by a signal void within it due to flowing blood, along with some thrombus. This complication can be distinguished with MR imaging from hematoma and thrombus by using phase display images to identify the motion of blood [23]. The Damus-Kaye-Stansel procedure [1 6, 1 7], which is done for palliation of severe obstruction to aortic blood flow, entails an anastomosis that is constructed between the end of the transsected proximal main pulmonary artery and the side of the intact ascending aorta. Blood flow to the lungs is maintamed by a shunt from the aorta to the distal pulmonary artery or its main branches. Stenosis of the anastomosis is the most
AJR:155,
August
MR OF CYANOTIC
1990
CONGENITAL
HEART
DISEASE
265
Transverse (A-C) and sagittal of patient with transposition of great arteries and Senning procedure. Baffle (arFig. 5.-A-D, (D) MR images
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rowbeads)
Is visualized
on images
A-C
extend-
ing craniad to caudad. Pulmonary veins (straight solid arrows) enter portion of atrium outside baftIe. Baffle redirects flow from superior and inferior vena cava Into systemic atrial chamber, which drains (straight open arrow) into left vontricle (LV). Note narrowing of superior limb of baffle on transverse (asterisk) and on sagittal (curved arrow) Images. RV = right ventricle.
C
common complication [24]. MR images in the transverse and coronal planes seem to be the most effective for depicting the anastomosis (Fig. 2). However, further experience is needed to assess the role of MR imaging in the evaluation of this procedure. The Jatene procedure [1 4, 1 5, 25], or arterial switch openation, is an anatomic correction of transposition of the great vessels performed by transsecting both great arteries and connecting the proximal pulmonary artery to the sinus portion of the aorta and the proximal aorta to the sinus portion of the pulmonary artery. MR images in the transverse and sagittal planes are effective to assess the great vessel morphology after the Jatene procedure. Postoperatively, the aorta is situated posterior to the main pulmonary artery and between the right and the left pulmonary artery, sometimes resulting in diffuse as well as focal narrowing at the origin of the right and left pulmonary arteries (Fig. 1). This is shown with MR imaging. Because arteries are small in patients who undergo this procedure in the neonatal period, thin sections (3 mm) are useful. Other complications of the Jatene procedure are obstruction of the right ventricular outflow region (Fig. 1 C) and/or the supravalvular portion of the new pulmonary artery at the site where the coronary arteries were removed, and
D
supravalvular aortic stenosis [14, 1 5, 25]. Coronary arterial stenosis or occlusion also may occur after the Jatene procedune, but the diagnosis of these complications is beyond the current capability of MR imaging. The Senning procedure [26] is used to correct transposition of the great arteries by creating a baffle that divides the atrium into a systemic and pulmonary atrial chamber, rerouting the blood from the superior and inferior vena cava to the left ventricle. The blood from the pulmonary veins is channeled into the right ventricle, thus reestablishing a normal circulatory route. The baffle can be visualized on transverse and sagittal MR images, and the most frequent complication, obstruction of the superior limb of the baffle near the connection to the superior vena cava, was shown with MR imaging in this study (Fig. 5) and in a prior report [27]. However, depiction of baffle leaks may not be recognized with spin-echo MR imaging. This study shows that MR imaging is effective in the postoperative evaluation of patients with cyanotic congenital heart disease. It may obviate angiography in many cases, but further experience is needed to establish this notion. The role of MR imaging relative to echocardiography has yet to be determined. This was not the focus of the current study, but will be interesting to evaluate in the future. MR imaging
KERSTING-SOMMERHOFF
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266
depicts the great vessels and the effectively as intracardiac morphology,
supracardiac region as which makes it partic-
ularly
of patients.
useful
in the current
population
MR
studies
of infants have become feasible with reduction of slice thickness to 3 mm without a critical decrease in signal-to-noise ratio. However, in critically ill patients needing life-sustaining equipment, MR imaging may not be a realistic consideration, whereas echocardiography is effective. Cine MR imaging [28, 29], especially the new velocity-encoded technique, may significantly broaden the spectrum of MR diagnoses already
possible
by adding
information
on valvular
lesions
DW, et al. Magnetic
resonance
and flow
characteristics.
REFERENCES 1 . Higgins
CB, Byrd
patients 2. Fletcher
with congenital BD, Jacobstein
BF, Farmer
imaging
heart disease. Circulation 1984;70:851-860 MD, Nelson AD, Riemenschneider TA, Alfidi
Gated magnetic resonance
imaging of congenital
in AJ.
cardiac malformations.
Radiology 1984;150:137-140 3. Dicier D, Higgins CB, Fisher MA, Osaki L, Silverman NH, Cheitlin MD. Congenital heart disease: gated MA imaging in 72 patients. Radiology 1986;158:27-235 4. Mirowitz SA, Gutierrez FA, Canter CE, Vannier MW. Tetralogy of Fallot: MR findings.Radiology 1989;171:207-212 5. Link KM, Hen-era MA, D’Souza VJ, Formanek AG. MA imaging of Ebstein anomaly: results in four cases. AiR 1988;150:363-367 6. Akins EW, Martin TD, Alexander JA, et al. MR imaging of double outlet right ventricle. AJR 1989;152:128-130 7. Kersting-Sommerhoff BA, Diethelm L, Teitel DF, et al. Magnetic resonance imaging of congenital heart disease: sensitivity and specificity using receiver operating characteristic curve analysis. Am Heart J 1989;1 88: 155-1 63 8. Kerstlng-Sommerhoff BA, Sechtem UP, Higgins CB. Evaluation of pulmonary blood supply by nuclear magnetic resonance imaging in patients with pulmonary atresia. J Am coil Cardiol 1988;1 1:166-171 9. Formanek AG, Witcofski AL, D’Souza VJ, Link KM, Karstaedt N. MR imaging of the central pulmonary arterial tree in conotruncal malformation. AJR 1986;147:1127-1131 10. Fletcher BD, Jacobstein MD. MAI of congenital anomalies of the great arteries. AJR 1986;146:941-948 11. von Schulthess GK, Higashino SM, Higgins 55, et al. Coarctation of the
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schneider TA. Magnetic resonance imaging: evaluation of palliative systemic-pulmonary shunts. circulation 1984;70:650-656 13. Soulen RL, Donner AM, Capitanio M. Postoperative evaluation of complex congenital heart disease by MRI. RadioGraphics 1987;7:975-1000 14. Jatene AD, Fontes VF, Paulista PP, et al. Anatomic correction of transposition of the great vessels. J Thorac Cardiovasc Surg 1976;72:364-370 15. Jatene AD, Fontes VF, Souza LCB, et al. Anatomic correction of transposition of the great vessels. J Thorac Cardiovasc Surg 1982;83:20-26 16. kaye MP. Anatomic correction of transposition of the great arteries. Mayo din Proc 1975;50:338-340 17. Stansel HC. A new operation for d-Ioop transposition of great vessels. Ann Thorac Surg 1975;19:565-567 18. Aastelli GO, McGoon DC, Wallace RB. Anatomiccorrection of transposition of great arteries with ventricular septal defect and subpulmonary stenosis. J Thorac Cardiovasc Surg 1969;58:545-552 19. Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax 1971; 26:240-248 20. Fontan F, Deville C, Quaegebeur J, et al. Repair of tricuspid atresia in 100 patients. J Thorac Cardiovasc Surg 1983;85:647-660 21 . Julsrud PA, Ehman AL, Hagler DJ, llstrup DM. Extracardiac vasculature in candidates for Fontan surgery: MA imaging. Radiology 1989;173:
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Girod DA, Fontan F, Deville C, Ottenkamp J, Choussat A. Long-term results after the Fontan operation for tricuspid atresia. Circulation 1987; 75:605-610 White EM, Edelmann AR, Weeden VJ, Brady TJ. Intravascular signal in MR imaging: use of phase display for differentiation of blood-flow signal from intraluminal disease. Radiology 1986;161 :245-249 Waldmann JD, Lamberti JJ, George L, et al. Experience with Damus procedure. Circulation [suppi Ill] 1988;78:32-39 Wemovsky G, Houghen TJ, Walsh EP, et al. Midterm results after the arterial switch operation for transposition of the great arteries with intact ventricular septum: clinical, hemodynamic, echocandiographic and electrophysiologic data. Circulation 1988;77: 1333-1344 Senning A. Surgical correction of transposition of the great vessels. Surgery 1959;45:966-980 Campbell AM, Moreau GA, Johns JA, et al. Detection of caval obstruction by MAI after intra-atrial repair of transposition of great arteries. Am J Cardiol 1987;60:688-691 Chung kJ, Simpson IA, Glass RF, et al. Cine MA after surgical repair in patients with transposition of great arteries. Circulation 1988;77: 104-109 Palo NJ, Shimakawa A, Glover GH. Phase contrast one MRI. Magn Reson Med 1989;1:101
259
Evaluation of Surgical Procedures for Cyanotic Congenital Heart Disease by Using MR Imaging
Barbara
A. Kersting-Sommerhoffi Klaus C. Seelos1 Christian Hardy2 Chisato Kondo1 Sarah S. Higgins2 Charles B. Higgins1
ECG-gated MR imaging has been shown to be effective for the diagnosis of congenital disease. In this study, we assessed its role in the postoperative evaluation of surgical procedures in patients with complex congenital heart disease. MR images of 26 patients with Rastelli (five), Fontan (three), Senning (three), Damus (one), Jatene (eight), Waterston (four), and Potts (two) procedures were evaluated retrospectively. The accuracy of MR imaging was compared with that of angiography in 20 patients. The surgical anastomoses were identified in all patients. Patency, atresia, or hypoplasia of central pulmonary arteries and postoperative complications (focal stenoses of pulmonary arteries, thrombosed conduit, peri-conduit abscess) were shown. Narrowing of the right ventricular outflow tract and focal compression of the proximal pulmonary arteries were recognized as specific complications of the Jatene procedure. MR imaging appears to be effective in the postoperative evaluation of surgical heart
procedures
used for congenital
heart disease.
It should
to repeated catheterization and angiography children with complex congenital heart disease.
AJR 155:259-266,
Aeceived February vision April 19, 1990. I
Department
16,
1990;
of Aadiology
accepted
(L308),
after reBox
0628,
University of California, San Francisco, Medical Center, San Francisco, CA 94143. Address reprint requests to C. B. Higgins. 2 Oakland Children’s Hospital, 51 05 Dover St., Oakland,
CA 94609.
0361 -803x/90/1 552-0259 Roentgen Ray Society
© American
August
be considered
for the
as an alternative
postoperative
examination
of
1990
Previous studies have established spin-echo MR imaging as an effective, noninvasive method for evaluating congenital disease of the heart [1 -7] and the great arteries [8-1 1]. When compared with echocardiography, one of the major advantages of MR imaging is its usefulness for evaluating great vessel abnormalities. Because a number of surgical procedures used in the treatment of cyanotic heart disease involve the great arteries or cardiovascular structures superior to the heart, MR imaging may be particularly useful in the assessment of these structures. Indeed, previous reports have shown the effectiveness of MR imaging for the evaluation of systemic to pulmonary shunts [12, 13]. The arterial switch (Jatene) procedure [14, 15], the Damus-Kaye-Stansel (Damus) procedure [16, 17], and the Rastelli procedure [1 8] are other surgical procedures involving structures above the base of the heart. The usefulness of MR imaging for evaluating these procedures has not yet been reported. The purposes of the current study were (1 ) to assess the effectiveness of spinecho MR imaging in the postoperative examination of patients with surgical procedures in the supracardiac region for the treatment of cyanotic congenital heart disease; (2) to compare the accuracy of MR imaging with that of angiography for identifying complications of the surgical procedures, including occlusion, stenosis, and aneurysm formation of conduits and anastomoses; and (3) to compare MR imaging with angiography for defining the status of the pulmonary arteries distal to various anastomoses and conduits. Subjects Twenty-six procedures
and Methods patients
(Rastelli,
with
congenital
five; Fontan, three;
heart
disease
Senning,
three;
and
palliative
Damus,
one;
or
corrective
Jatene,
eight;
surgical Water-
260
KERSTING-SOMMERHOFF
ston,
four;
imaging.
and
two)
description
underwent
ECG-gated,
of the surgical
procedures
spin-echo and
their
is given
to 28 years
patients
who
congenital
Images ing
were
were
magnet
ECG
acquired
was
in the transverse were
surgical
by using MT/S,
an echo
the AR interval images
after
a cryogenic
Milpitas,
(General
delay
applied
time
plane
in all patients; were
CA)
Electric (TE)
and thus depended acquired
procedures
for
0.35-T or
MR images reviewers
Signa,
of 25-30 the repetition
on the patient’s
obtained
depending
in all patients; on the
type
were time
anastomosis; WI).
obtained.
or coronal procedure
(in 17 and nine patients, of both
respectively). Images with a slice thickness 5 and 10 mm were obtained in 19 patients. In the last patients
TABLE
1: Surgical Procedure
Rastelli
did
of
not
retrospectively the
know
general the
by three reviewers.
diagnosis
results
of
other
definition
of the
central
pulmonary
Operative
Description from
Corrected
Lesion
RV to MPA
Pulmonary
atre-
sia, severe pulmonary stenosis Fontan
Conduit
or direct
anasto-
Tricuspid atresia, severe tricuspid ste-
of right atrial ap-
mosis pendage
to MPA
nosis, Senning
Surgically
created
baffle
teries
nary atrial chambers End-to-side anastomosis of MPA to ascending
Ao; shunt
Severe stenosis of LVOT; ob-
from Ao to
structing VSD in tncuspid atresia Transposition of great antenies
MPA
Jatene
Transsection
of great
ar-
teries above sinuses and switching of arteries to opposite
nuses:
Potts
hypo-
plastic RV Transposition of great ar-
dividing the atrium into systemic and pulmoDamus
si-
reimplantation
of coronary arteries from aortic sinuses to the switched ascending Ao Side-to-side anastomosis
of descending left PA
Pulmonary
Ao to
nosis
ste-
(tetnal-
ogy
of Fallot), pulmonary
atnesia, cuspid sia Waterston
Side-to-side
of ascending right
tnatne-
Pulmonary
anastomosis
Ao to
nosis
ogy
PA
ste-
(tetnalof Fallot),
pulmonary atnesia, tncuspid sia
=
Note.-RV ventricular
= right ventricle; MPA = main pulmonary septal defect; PA = pulmonary artery.
the
imaging
The
surgical studies
features: demof the site of
arteries;
Procedures
Conduit
and
and
identi-
fication of postoperative complications at valvar, anastomotic, and arterial levels. Successful studies had to meet the following criteria: depiction of the shunt or baffle on at least two images or in two different planes and direct visualization of the systemic-pulmonary arterial anast#{243}mosis. Patency of a shunt or conduit was determined by the absence of signal in the vessel lumen. The MR results were compared with the findings at surgery and angiography. Twenty of the patients had angiography to corroborate MR findings. In these 20 patients, MR and angiographic findings were
1 .5-T
(TR) equaled
sagittal
but
aware
or angiography). The MR images were evaluated for the following onstration of the shunt, conduit, or baffle; depiction
heart rate. Images
of surgical
1990
(echocardiography
superconductMilwaukee,
msec
were evaluated
were
procedure,
complex
a cryogenic
August
patients.
disease.
magnet
with gating
imaged heart
(Diasonics
superconducting Images
in Table
(mean,
cyanotic
AJR:155,
imaged who had had Jatene procedures, images of 3-mm thickness were acquired. In seven older children and adults, the slice thickness was 1 0 mm. Angiographic studies were available in 20 of the 26
MR major
1 . Patients ranged in age from 6 months 7 years). Twelve children were 3 years old or younger. Children under 6 years old were sedated 30 mm before the examination with 100 mg/kg chloral hydrate (maximum total dose, 1 500 mg) orally or rectally, or nembutal 5 mg/kg (maximum dose, 1 00 mg) intramuscularly. The subjects of the study were consecutive applications
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Potts,
A brief
ET AL.
artery;
Ao
=
aorta;
LVOT
=
atne-
left ventricular
outflow
tract;
VSD
AJR:155,
August
MR OF CYANOTIC
1990
CONGENITAL
compared. In the remaining patients, echocandiognaphy and surgical findings were used to corroborate the findings of MR imaging.
HEART
261
DISEASE
the one remaining case, a diminutive pulmonary artery was found during autopsy. All other pulmonary arterial segments were found to be normal with both techniques.
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Results Demonstration
of Anastomoses
with MR
Complications
The shunts, conduits, or baffles were successfully imaged in all 26 patients. In all 26 cases, the anastomoses could be visualized directly on transverse images. Waterston, Potts, Fontan, and Damus procedures were best visualized on transverse images: images in the coronal plane generally did not provide important additional information. Rastelli and Jatene procedures were depicted on transverse and sagittal images; images in the sagittal plane were especially valuable for detecting postoperative complications (Fig. 1). In all but four cases, the surgical connection could be seen on more than one image; in these four cases, the slice thickness was 1 0 mm. The Rastelli, Senning, and Damus procedures were visualized longitudinally on images in the sagittal or coronal plane (Figs. 2 and 3).
Comparison Central
of MR and Angiography
Pulmonary
Arteries
Pulmonary arterial segments of 20 patients were analyzed and compared with angiographic findings, with consideration of the main, left, and right pulmonary arteries as separate segments (Table 2). Three right and two left pulmonary arteries were diagnosed as stenotic on both MR and angiography. Eight pulmonary arterial segments (one right, one left, six main) were not visualized with MR imaging or angiography. On review of the patients’ surgical or autopsy reports, these vessels proved to be atretic in seven of eight segments. In
Fig. 1.-Adjacent transverse MR images of a patient A, Aorta (A) is located posterior to main pulmonary
Table 3 summarizes the number of complications evaluated by MR and angiography and the number of complications identified by the two techniques. Complications were seen in all four patients who had undergone the Rastelli procedure. In two patients, narrowing of the conduit was identified; in the third, a thrombus obstructing the conduit was detected with MR imaging. One of these patients had a stenosed right pulmonary arterial segment also. The fourth patient, with clinical symptoms of endocarditis, had an intramural abscess within the right ventricular wall (infectious pseudoaneurysm) and surrounding the conduit. Two of these patients were studied with angiography, and the complications were detected in both. The complications in all four patients were confirmed during subsequent surgery. Four complications of Fontan procedures were seen by MR in two different patients. However, only two of the four complications were identified by angiography. Considerable right atrial enlargement, with the atrial septum bulging into the left atrium, was present in both patients. This enlargement compressed the entrance of the right pulmonary veins into the left atrium. A thrombus adhering to the atrial septum was shown with MR in one of these patients. The bulging septum and compression of the ostium of the right pulmonary vein was confirmed with angiography, but the thrombus was not identified. The other patient had a cardiac mass that was diagnosed with MR imaging as a partially thrombosed pseu-
with transposition of great arteries and Jatene procedure. artery (P) and compresses proximal right (RP) and left (LP) pulmonary
arteries (arrows
= focal
stenoses). B, Aortic root (AR) is dilated and right ventricular outflow tract (asterisk) is hypertrophied. C, Sagittal MR image of a different patient who had undergone same procedure. Note severe carotid artery, A = aorta, asterisk = RV outflow tract, RV = right ventricle, LV = left ventricle.
right ventricular
outflow
tract
stenosis
(black
arrow).
C =
262
KERSTING-SOMMERHOFF
ET AL.
AJR:155,
Fig.
2.-A-D,
August
1990
(A, B) and coronal of patient with malaligned
Transverse
(C, D) MR images
atrioventricular canal and subaortic stenosis treated with Damus procedure (end-to-side anastomosis of proximal main pulmonary artery
[P] and ascending
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aorta [A]). Wide communica-
two vessels is clearly shown in both planes (A, C). B depicts narrowing of left ventricular outflow tract (curved arrow) as well
tion
between
as left and right ventricular hypertrophy. Note pulmonary veins entering left atrium (LA). Small arrows on A are pulmonary valve leaflets. RV = right ventricle, LV = left ventricle, RA = right atrium, I = innominate artery, C = carotid artery.
doaneurysm measuring 6 cm in diameter and arising from the right ventricle (Fig. 4). The presence of a pseudoaneurysm was not diagnosed with angiography but was confirmed at subsequent surgery. MR imaging and angiography in a patient with the Senning procedure showed stenosis at the superior limit of the baffle, causing superior vena caval obstruction (Fig. 5). Another patient had pulmonary arterial hypertension after surgery. This was shown on MR images by enlarged pulmonary arteres and an elliptical configuration of the left ventricle, rather than the crescentic shape usually observed after this procedune. The ventricular septum in this patient was bowed toward the right ventricle, which is the reverse of the usual appearance in patients with transposition of the great arteries. In the patient with the Damus procedure, MR imaging identified a thrombus obstructing the left pulmonary artery (Fig. 2). This complication was not detected on angiography; the angiographic finding was considered as poor opacification of the left pulmonary artery for unknown reasons. The thrombus appeared as an area of high signal intensity completely obstructing the lumen of the left pulmonary artery. The thrombus was removed at subsequent surgery, and a follow-up
MR study showed no residual obstruction of the pulmonary artery. The eight patients with Jatene procedures had 1 5 morphologic abnormalities that were all shown with MR imaging (Table 1) (Fig. 1). Dilatation of the aortic root (three patients), supravalvar aortic stenosis (one patient), and narrowing of the right ventricular outflow tract (six patients) were depicted on MR images in all cases. These were confirmed with angiography in the four patients who had this study. The remainder had identical findings on echocardiography. Proximal narrowing of the right and left pulmonary arteries as they coursed around the repositioned aorta was identified in six patients on transverse MR images (Fig. 1). There was complete agreement between MR imaging and angiography with regard to the nine abnormalities evaluated by both techniques. No complications were detected in the six patients with Waterston or Potts shunt.
Discussion This study assessed the echo MR imaging to evaluate
capability of ECG-gated, spinsurgical procedures used in the
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AJR:155,
August
MR OF CYANOTIC
1990
CONGENITAL
HEART
263
DISEASE
Fig. 3.-A-D, Transverse (A-C) and sagittal (D) MR images of patient with truncus arteriosus and uncomplicated Rastelli procedure. On transverse images, conduit (C) can be followed from right ventricle (RV) to its entrance into main pulmonary artery (P). Conduit is depicted longitudinally on sagittal image. A = aorta, LA left atrium, RA = right atrium, LV = left ventricle, RP = right pulmonary artery, LP = left pulmonary artery.
, ,
i.;i; I”-.’
‘u..J
C TABLE
2: Status
of Pulmonary Right (n =
Nonmal Stenotic Atnetic Note-PA
=
Arterial
PA
D (n
Segments
Left PA (n = 20)
20)
= 60)
Main (n =
PA
20)
MR
Angio
MR
Angio
MR
Angio
16 3 1
16
17
17
14
14
0 6
0 6
pulmonary
artery.
3 1
2 1 Angio
2 1 =
angiography.
treatment of cyanotic congenital heart disease. In comparison with angiography, the results show that MR imaging can reliably depict the anatomy of the surgical procedures, postoperative complications, and the status of the central pulmonary arteries. MR imaging is as effective as angiography for the identification of these complications and of the status of the pulmonary arteries. The results suggest that MR imaging could be used to monitor size, patency, and eventually the growth of the pulmonary arteries under improved perfusion conditions after construction of systemic-pulmonary shunts. In an earlier study, Jacobstein et al. [1 2] reported the usefulness of ECG-gated MR for the evaluation of systemic-
pulmonary artery shunts in a study population that consisted mostly of patients with Blalock-Taussig and Glenn shunts. The current study focused on patients with Rastelli, Fontan, and Senning procedures, and two of the newer surgical techniques, namely Damus and Jatene procedures, for connection on palliation of cyanotic congenital heart disease. In patients who underwent a Rastelli procedure, MR imaging displayed the conduit between the right ventricle and the pulmonary artery (Fig. 3) in the transverse and sagittal planes. The sagittal plane was found to be the most effective for showing the proximal anastomosis to the right ventricle and the distal anastomosis to the pulmonary artery. Narrowing of the conduit, stenosed origins of the right or left pulmonary arteries, and pseudoaneurysm were complications depicted with MR imaging. Compression of the graft by the sternum or other structures in an excessively narrowed space anterior to the right ventricle can be detected on sagittal MR images. Demonstration of valvular obstruction is limited with spinecho MR imaging. The Fontan procedure creates an anastomosis between the right atrium and pulmonary artery; it can generally be done either directly between the right atrial appendage and pulmonary artery or with graft material between the right
KERSTING-SOMMERHOFF
264
TABLE
3: Complications:
Comparison
ET AL.
AJR:155,
1990
of MR and Angiography
MR Imaging
Angiognaphy
Procedure
Specific Complications No. Studied
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August
No. Identified
No. Studied
No. Identified
Rastelli
4
4
2
2
Fontan
4
4
4
2
Senning Damus
1 1
1 1
1 1
1 0
15
15
9
9
Narrowing of conduit (n = 2), thnombosed conduit (n = 1), peniconduit abscess (n = 1) Aneunysmal dilatation of RA (n = 2), RA thnombus (n = 1), RV pseudoaneunysm(n=1)
Jatene
Note.-AA
=
right atrium;
RV
=
right ventricle;
PA
=
pulmonary
artery;
AVOT
Baffle stenosis (n = 1) Thrombus in left PA (n = 1) Aortic dilatation (n = 3), supravalvular aortic stenosis (n = 1), narrowing of RVOT (n = 6), proximal PA stenosis (n = 6) =
right ventricular
outflow
tract.
Fig. 4.-A-C, Transverse MR images of patient with tricuspid atresia and Fontan procedure. Right atrium (RA) is markedly enlarged, with atrial septum (arrowhead) bulging toward left and compression of entrance of pulmonary veins (arrow) into left atrium (LA). Conduit (C) originates from right atrium and enters the pulmonary artery (P). Phase-encoded imaging indicated slow flowing blood within a partially thrombosed pseudoaneurysm (asterisk) anterior to right ventricle. A = aorta.
atrium and pulmonary artery [1 9, 20]. Infrequently a valve may be included in the anastomosis. One recent report has described the effectiveness of MR imaging for the postoperative evaluation of the Fontan procedure [21 ], showing patency of the anastomosis and the size of the central and hilar pulmonary arteries. Complications [22] of this operation indude conduit obstruction, residual atrial septal defects, and systemic venous hypertension. Residual septal defects, however, may not be recognized with MR imaging and require Doppler or color flow echocardiography or angiography for detection and quantification. In the current study, an unusual complication, a pseudoaneurysm surrounding the anastomo-
sis, was characterized by a signal void within it due to flowing blood, along with some thrombus. This complication can be distinguished with MR imaging from hematoma and thrombus by using phase display images to identify the motion of blood [23]. The Damus-Kaye-Stansel procedure [1 6, 1 7], which is done for palliation of severe obstruction to aortic blood flow, entails an anastomosis that is constructed between the end of the transsected proximal main pulmonary artery and the side of the intact ascending aorta. Blood flow to the lungs is maintamed by a shunt from the aorta to the distal pulmonary artery or its main branches. Stenosis of the anastomosis is the most
AJR:155,
August
MR OF CYANOTIC
1990
CONGENITAL
HEART
DISEASE
265
Transverse (A-C) and sagittal of patient with transposition of great arteries and Senning procedure. Baffle (arFig. 5.-A-D, (D) MR images
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rowbeads)
Is visualized
on images
A-C
extend-
ing craniad to caudad. Pulmonary veins (straight solid arrows) enter portion of atrium outside baftIe. Baffle redirects flow from superior and inferior vena cava Into systemic atrial chamber, which drains (straight open arrow) into left vontricle (LV). Note narrowing of superior limb of baffle on transverse (asterisk) and on sagittal (curved arrow) Images. RV = right ventricle.
C
common complication [24]. MR images in the transverse and coronal planes seem to be the most effective for depicting the anastomosis (Fig. 2). However, further experience is needed to assess the role of MR imaging in the evaluation of this procedure. The Jatene procedure [1 4, 1 5, 25], or arterial switch openation, is an anatomic correction of transposition of the great vessels performed by transsecting both great arteries and connecting the proximal pulmonary artery to the sinus portion of the aorta and the proximal aorta to the sinus portion of the pulmonary artery. MR images in the transverse and sagittal planes are effective to assess the great vessel morphology after the Jatene procedure. Postoperatively, the aorta is situated posterior to the main pulmonary artery and between the right and the left pulmonary artery, sometimes resulting in diffuse as well as focal narrowing at the origin of the right and left pulmonary arteries (Fig. 1). This is shown with MR imaging. Because arteries are small in patients who undergo this procedure in the neonatal period, thin sections (3 mm) are useful. Other complications of the Jatene procedure are obstruction of the right ventricular outflow region (Fig. 1 C) and/or the supravalvular portion of the new pulmonary artery at the site where the coronary arteries were removed, and
D
supravalvular aortic stenosis [14, 1 5, 25]. Coronary arterial stenosis or occlusion also may occur after the Jatene procedune, but the diagnosis of these complications is beyond the current capability of MR imaging. The Senning procedure [26] is used to correct transposition of the great arteries by creating a baffle that divides the atrium into a systemic and pulmonary atrial chamber, rerouting the blood from the superior and inferior vena cava to the left ventricle. The blood from the pulmonary veins is channeled into the right ventricle, thus reestablishing a normal circulatory route. The baffle can be visualized on transverse and sagittal MR images, and the most frequent complication, obstruction of the superior limb of the baffle near the connection to the superior vena cava, was shown with MR imaging in this study (Fig. 5) and in a prior report [27]. However, depiction of baffle leaks may not be recognized with spin-echo MR imaging. This study shows that MR imaging is effective in the postoperative evaluation of patients with cyanotic congenital heart disease. It may obviate angiography in many cases, but further experience is needed to establish this notion. The role of MR imaging relative to echocardiography has yet to be determined. This was not the focus of the current study, but will be interesting to evaluate in the future. MR imaging
KERSTING-SOMMERHOFF
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266
depicts the great vessels and the effectively as intracardiac morphology,
supracardiac region as which makes it partic-
ularly
of patients.
useful
in the current
population
MR
studies
of infants have become feasible with reduction of slice thickness to 3 mm without a critical decrease in signal-to-noise ratio. However, in critically ill patients needing life-sustaining equipment, MR imaging may not be a realistic consideration, whereas echocardiography is effective. Cine MR imaging [28, 29], especially the new velocity-encoded technique, may significantly broaden the spectrum of MR diagnoses already
possible
by adding
information
on valvular
lesions
DW, et al. Magnetic
resonance
and flow
characteristics.
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in AJ.
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